Clinical Spectrum, Quality of life, BRAF Mutation Status and Treatment of Skin Involvement in Adult Langerhans Cell Histiocytosis.

This study highlights the need to evaluate quality of life and to screen for associated malignancy in adult patients with Langerhans cell histiocytosis. PMID: 28421232 [PubMed - as supplied by publisher]
Source: Acta Dermato-Venereologica - Category: Dermatology Authors: Tags: Acta Derm Venereol Source Type: research

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ConclusionsIdiopathic granulomatous hypophysitis, a rare inflammatory disease of the pituitary gland, is a diagnosis of exclusion for which both medical and surgical management are reported in the literature. We present a case of idiopathic granulomatous hypophysitis who was symptom free with no complications of hypopituitarism following its transsphenoidal resection after 9  months of follow-up.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
AbstractSinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are often nonspecific, potentially mimicking malignancies. The diagnosis is ultimately made based on pathology, in which the lymph nodes are characterized by a dilated subcapsular sinus filled with histiocytes that can exhibit emperipolesis. Immunohistochemically, the histiocytes are variably CD68 positive and reliably negative for CD1a. The features of head and neck sinus histiocytosis with massive lymphadenopathy are exemplified in this radiology-pathol...
Source: Head and Neck Pathology - Category: Pathology Source Type: research
We report the case of a 55-year-old adult who presented with a single lytic self-healing lesion of the skull, invading adjacent soft tissues. The histology and cytology were also typical of LCH, and tumor cells contained the BRAFV600E mutation. However, histiocytes were negative for CD1a and CD207. We suggest that this case might be considered as LCH, despite its abnormal phenotype.
. PMID: 31661070 [PubMed - as supplied by publisher]
Source: Clinical Neuropathology - Category: Pathology Authors: Tags: Clin Neuropathol Source Type: research
No abstract available
Source: Journal of Thoracic Imaging - Category: Radiology Tags: Web Exclusive Content: JSTR Meeting Notes Source Type: research
In this report, we present a young infant with multisystem Langerhans cell histiocytosis, who after cladribine and cytarabine salvage treatment developed immune thrombocytopenia (IT). On review of the literature, there were no previous reports of Langerhans cell histiocytosis–associated IT. Treatment of the IT with intravenous immunoglobulin and oral corticosteroids was unsuccessful. Eltrombopag, in combination with a 4-day course of dexamethasone was commenced as second-line therapy. Platelet recovery occurred 10 days after initiation of eltrombopag. The immune thrombocytopenia remains in long-term remission despite...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research
In conclusion, idMTX is associated with better EFS. DP on induction remains of dismal prognosis in relation to disease REA afterwards. Risk stratification should highlight the role of trihemopoietic cytopenias, hepatic dysfunction, tri RO+, central nervous system risk site, and lung association.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research
Pneumothorax may recur during pulmonary Langerhans cell histiocytosis (PLCH) patients ’ follow-up and its management is not standardised. The factors associated with pneumothorax recurrence are unknown.
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Valentin H Abstract OBJECTIVE: Langerhans cell histiocytosis (LCH) is a granulomatous inflammatory myeloid neoplasia associated with a cytokine storm in both serum and lesions. Increased levels of plasma interleukin-17A (IL-17A) in LCH patients have been reported, but this finding was not confirmed in all studies. Neurodegeneration is a devastating complication of LCH (ND-LCH). We aimed to revisit the issue of plasma IL-17A levels in LCH, by using a larger number of patients, and also to investigate the relationship between IL-17A and LCH sequelae, especially ND-LCH. METHODS: Plasma samples from 68 LCH patien...
Source: Cytokine - Category: Molecular Biology Authors: Tags: Cytokine Source Type: research
The objective of this literature review is to present the theoretical and practical aspects of extracorporeal membrane oxygenation support for respiratory and/or cardiac functions in critically ill patients.
Source: Revista Brasileira de Terapia Intensiva - Category: Intensive Care Source Type: research
Abstract Diffuse cystic lung diseases (DCLDs) are a group of diverse pulmonary disorders with varying pathophysiology that are characterized by the presence of thin-walled, air-filled spaces within lung parenchyma. High-resolution computed tomography plays a crucial role in the evaluation of DCLDs, and cyst characteristics such as morphology, distribution, and the presence of other associated radiologic findings can help distinguish between different DCLDs. DCLDs can be classified according to their underlying pathophysiology as neoplastic, genetic, lymphoproliferative, infectious, associated with other forms of i...
Source: Respiratory Care - Category: Respiratory Medicine Authors: Tags: Respir Care Source Type: research
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